Reduction of Antibiotic-Associated Conditions After Tympanostomy Tube Placement in Children.

OBJECTIVE: Tympanostomy tube placement has been shown to decrease systemic antibiotics usage in patients with recurrent acute otitis media. Systemic antibiotics in children are associated with an increase in antibiotic-associated conditions (asthma, allergic rhinitis, food allergy, atopic dermatitis, celiac disease, overweight/obesity, attention-deficit hyperactivity disorder [ADHD], autism, learning disability, and Clostridium difficile colonization) later in life. The objective of this study is to estimate whether tympanostomy tube placement is associated with a reduction in antibiotic-associated conditions in children with recurrent acute otitis media (RAOM). METHODS: A retrospective cohort review of electronic medical records from 1991 to 2011 at a large pediatric hospital system was performed identifying 27,584 patients under 18 years old with RAOM, defined by 3 or more episodes of AOM. Antibiotic-associated conditions were defined using ICD-9 and ICD-10 codes. RESULTS: The enrollment population was largely composed of White patients (28.9%), Black patients (30.1%), and Hispanic/Latino patients (16.4%). The number of systemic antibiotics prescribed per encounter was significantly lower in children who pursued tympanostomy tubes (0.14 antibiotics per encounter) versus those who did not (0.23 antibiotics per encounter) (p < 0.001). Patients with RAOM who received tympanostomy tubes were less likely to have diagnoses of overweight/obesity (OR. 0.62 [0.55, 0.68]; p < 0.001), asthma (OR 0.8 [0.74, 0.87]; p < 0.001), allergic rhinitis (OR 0.72 [0.65, 0.81]; p < 0.001), and atopic dermatitis (0.78 [0.71, 0.86]; p < 0.001). CONCLUSIONS AND RELEVANCE: Tympanostomy tube placement is associated with less systemic antibiotic administration and a decreased incidence of overweight/obesity, asthma, allergic rhinitis, and atopic dermatitis in children diagnosed with RAOM. LEVEL OF EVIDENCE: 4 Laryngoscope, 2024.

Use of External Distractors and the Role of Imaging Prior to Mandibular Distraction in Infants With Isolated Pierre Robin Sequence and Stickler Syndrome.

IMPORTANCE: Computed tomographic (CT) scans are often obtained before mandibular distraction osteogenesis in patients with isolated Pierre Robin sequence. There is concern regarding the risk of radiation exposure from CT in children. OBJECTIVE: To evaluate whether preoperative CT is necessary for adequate airway, feeding, and aesthetic outcomes following mandibular distraction with external distraction devices in infants with isolated Pierre Robin sequence. DESIGN, SETTING, AND PARTICIPANTS: In a retrospective review of medical records, infants who underwent mandibular distraction between January 1, 1998, and September 30, 2014, at 2 tertiary children's hospitals were identified using procedure codes. Data analysis was conducted December 1, 2014, to March 31, 2015. Fifty-two patients fit the inclusion criteria of isolated Pierre Robin sequence or Stickler syndrome, of being younger than 9 months at the time of distraction, and of use of external distractors. Forty-two of these infants did not receive preoperative CT imaging. EXPOSURE: Mandibular distraction osteogenesis for isolated Pierre Robin sequence or Stickler syndrome. MAIN OUTCOME MEASURES: Number of infants who were able to avoid tracheostomy or achieve decannulation, who were able to avoid placement or achieve removal of a gastrostomy tube, and in whom there were no intraoperative complications, no open-bite deformity, no malocclusion, no asymmetry, and no postoperative complications. RESULTS: In comparison with the 10 infants who underwent preoperative CT, all 42 of the infants (100%) who did not receive preoperative CT imaging successfully avoided tracheostomy or achieved decannulation (P = .04) and 40 patients (95%) did not require placement of a gastrostomy tube or were able to undergo gastrostomy tube removal postoperatively (P < .001). There were no significant differences between the CT and non-CT groups in the other 5 outcome measures. Two patients (5%) required postoperative gastrostomy tube placement, 2 patients (5%) had minor intraoperative complications that might have been anticipated with CT, 2 patients (5%) demonstrated malocclusion, and 1 infant (2%) had asymmetry at the end of the distraction phase. CONCLUSIONS AND RELEVANCE: This series suggests that the absence of preoperative CT does not compromise functional or aesthetic outcomes in mandibular distraction with external distraction devices in infants with isolated Pierre Robin sequence or Stickler syndrome. This finding has implications for cost containment and reduction of radiation exposure to a vulnerable population. LEVEL OF EVIDENCE: 4.

Tramadol versus codeine/acetaminophen after pediatric tonsillectomy: A prospective, double-blinded, randomized controlled trial.

OBJECTIVE: Tonsillectomy is one of the most common pediatric surgical procedures performed in the United States. The postoperative period can be particularly painful, and there is currently no consensus on an optimal analgesic regimen. The objective of this study was to evaluate efficacy and safety of the single drug tramadol versus codeine/acetaminophen post-tonsillectomy. DESIGN: Prospective, double-blinded, randomized controlled trial. SETTING: Large, Midwestern US pediatric hospital. PATIENTS: Eighty-four children aged 4-15 years who underwent a tonsillectomy (with or without adenoidectomy) procedure were randomized and 74 were included in the analysis. INTERVENTIONS: Group 1 received liquid codeine/acetaminophen for 10 days post-tonsillectomy (5 days scheduled, followed by 5 days as-needed). Group 2 received liquid tramadol for 10 days post-tonsillectomy (5 days scheduled, followed by 5 days as-needed). MAIN OUTCOME MEASURES: Efficacy and side effects were evaluated using a 10-day take-home diary that was completed by parents. RESULTS: Children in both study arms reported adequate post-tonsillectomy pain management without significant differences between groups in pain scores. Oversedation was significantly higher on the day of surgery in the codeine/acetaminophen group, and itching was experienced by significantly more children in the tramadol group during the postoperative period. CONCLUSIONS: As part of multimodal analgesia, scheduled plus as-needed tramadol may be considered for children in the postoperative setting due to its analgesic properties, low potential for side effects, and good safety profile.

Surgery for pediatric invasive fungal sinonasal disease.

OBJECTIVES/HYPOTHESIS: To evaluate the management and outcomes of children with invasive fungal sinonasal disease treated with radical surgery. STUDY DESIGN: Retrospective case series. METHODS: From 1994 to 2007, 11 pediatric patients were identified with invasive fungal sinonasal disease treated surgically by the same pediatric otolaryngologist. Collected data included demographics, oncologic diagnoses, absolute neutrophil counts, symptoms, computed tomography scan findings, biopsy and culture results, surgical procedures, concurrent medical therapies, complications, and survival. RESULTS: The studied patient population consisted of four males and seven females with an average age of 10 years (range, 2-14 years). Six patients were diagnosed with acute lymphoblastic leukemia and five with acute myeloid leukemia, which included 10 cases of relapsed disease. The average number of severely neutropenic days prior to diagnosis of an invasive fungal infection was 18 (range, 8-41 days). Culture results demonstrated Alternaria in seven patients and Aspergillus in four. Nine patients underwent an external medial maxillectomy, five of which were bilateral, and six underwent septectomy. All 11 patients (100%) were cured of their invasive fungal sinonasal disease without relapse. Three patients eventually died from unrelated causes. CONCLUSIONS: Invasive fungal sinonasal disease is a life-threatening problem in immunocompromised children, especially with relapsed leukemia. Successful treatment depends on timely and aggressive surgical, antifungal, and supportive therapies. To our knowledge, this study represents the largest series of pediatric patients with invasive fungal sinonasal disease managed via an aggressive surgical approach with the best outcomes to date. LEVEL OF EVIDENCE: 4.

Outcomes of combined Furlow palatoplasty and sphincter pharyngoplasty for velopharyngeal insufficiency.

OBJECTIVE: To compare surgical outcomes between pharyngeal flap, sphincter pharyngoplasty, and combined Furlow palatoplasty and sphincter pharyngoplasty in the management of pediatric velopharyngeal insufficiency. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care pediatric hospital. SUBJECTS AND METHODS: After exclusion of children with velocardiofacial syndrome, 96 patients who underwent surgical intervention between 2008 and 2012 were identified. Surgical interventions were categorized as pharyngeal flap, sphincter pharyngoplasty, and combined Furlow palatoplasty and sphincter pharyngoplasty. Main outcome measures included perceptual speech analyses, complications, and surgical revision rates. RESULTS: Of the 96 reviewed patients, 38 (39.6%) underwent pharyngeal flap, 20 (20.8%) sphincter pharyngoplasty, and 38 (39.6%) combined Furlow palatoplasty and sphincter pharyngoplasty. Choice of surgical intervention was based on patient characteristics, observed palatal length, and formal speech assessments. There were no differences in patient demographics or preoperative perceptual speech analysis scores among the 3 surgical groups. The mean speech improvement was significantly greater in both the pharyngeal flap (P = .031) and combined procedure (P = .013) compared with sphincter pharyngoplasty alone, but no differences were observed between the pharyngeal flap and combined procedure (P = .797). There were no differences in complications among the 3 surgical interventions (P = .220). The combined procedure required significantly less surgical revisions than the pharyngeal flap (P = .019). CONCLUSION: Combined Furlow palatoplasty and sphincter pharyngoplasty is an effective procedure for the management of pediatric velopharyngeal insufficiency and may result in superior speech outcomes and lower revision rates than sphincter pharyngoplasty and pharyngeal flap, respectively.

The effect of cleft palate repair technique on hearing outcomes in children.

OBJECTIVE: Otitis media with effusion causing conductive hearing loss is a problem for many children with cleft palate. This study examines the association between palate repair technique and hearing outcomes in children at 3 and 6 years post-repair. PATIENTS AND METHODS: Retrospective chart review of patients with all types of cleft palate that were repaired between 2001 and 2006 at a tertiary children's hospital. Exclusion criteria included sensorineural hearing loss, ossicular chain abnormalities, and ear canal abnormalities. The primary outcome was pure tone average (PTA) from 0.5 kHz to 2 kHz. RESULTS: 69 patients (138 ears) were analyzed. 30.4% of left ears and 31.9% of right ears had an abnormal (>20 dB) PTA at 3 years; at 6 years this significantly improved to 13.0% (p=0.008) and 15.9% (p=0.011). Double-reverse z-plasty was associated with the lowest median PTA of 10.0 dB (p=0.046) at 6 years. There was no difference in median PTA between children with and without comorbid diagnoses (such as Pierre Robin Sequence, arthrogryposis) at either 3 years or 6 years (p=0.075, p=0.331). Multivariate model showed that extent of cleft influenced technique choice (p=0.027), but only technique choice was associated with significant differences in PTA and only at 6 years post-repair. CONCLUSION: The majority of children developed normal hearing by 6 years with palatoplasty and routine tube insertion. Double reverse z-plasty was associated with the best outcome, but is not ideal for hard palate clefts. Randomized controlled trials are needed to elucidate the relationship between technique, middle ear ventilation and time to recovery, irrespective of type of cleft.

Epidermal growth factor receptor inhibitor therapy for recurrent respiratory papillomatosis.

The epidermal growth factor pathway has been implicated in various tumors, including human papillomavirus (HPV) lesions such as recurrent respiratory papillomatosis (RRP). Due to the presence of epidermal growth factor receptors in RRP, epidermal growth factor receptor (EGFR) inhibitors have been utilized as adjuvant therapy. This case series examines the response to EGFR inhibitors in RRP. Four patients with life-threatening RRP were treated with EGFR inhibitors. Operative frequency and anatomical Derkay scores were calculated prior to, and following EGFR inhibitor treatment via retrospective chart review. The anatomical Derkay score decreased for all four patients after initiation of EGFR inhibitor therapy. In one patient, the operative frequency increased after switching to an intravenous inhibitor after loss of control with an oral inhibitor. In the other patients there was a greater than 20% decrease in operative frequency in one and a more than doubling in the time between procedures in two.  This study suggests that EGFR inhibitors are a potential adjuvant therapy in RRP and deserve further study in a larger number of patients.

Predicting surgical outcomes in pediatric cervicofacial nontuberculous mycobacterial lymphadenitis.

OBJECTIVES: We examined surgical outcomes in children with cervicofacial nontuberculous mycobacterial lymphadenitis and attempted to identify predictors of complications. METHODS: A retrospective chart review from 2 tertiary pediatric centers was used to identify .11 presentation or operative variables (age at surgery, gender, symptom duration, pain, violaceous skin changes, skin breakdown, fluctuance, purified protein derivative positivity, operative procedure, use of nerve integrity monitoring, and use of skin flap advancement) and to compare these to 5 postoperative complications (facial nerve dysfunction [paresis or paralysis], poor scarring, recurrence, wound infection, and wound dehiscence without infection). RESULTS: The 45 patients analyzed for presentation or operative variables (28 female, 17 male; average age, 31.2 months) typically presented with painless masses averaging 8.2 weeks in duration, along with violaceous skin changes in 29 of the 45 cases (64%) and skin breakdown in 9 cases (20%). The surgical procedures included parotidectomy with or without selective lymphadenectomy in 38 of the 45 cases (84%) and lymphadenectomy alone in 7 cases (16%). Skin resection and cervicofacial advancement flap reconstruction was performed in 20 cases (44%). Nerve integrity monitoring was utilized in 32 cases (71%). In the 44 patients analyzed for postoperative complications, we found facial nerve paresis in 14 (31.8%), poor scarring in 9 (20.5%), wound infection in 6 (13.6%), recurrence in 4 (9.1%), and facial nerve paralysis in 2 (4.5%). Nine of the 14 cases (64.3%) of initial facial nerve paresis resolved. At final follow-up, facial nerve paresis persisted in 5 of the 14 children (35.7%) with initial postoperative paresis and in 1 of the 2 children (50.0%) with initial postoperative paralysis. Facial nerve paralysis persisted in the other child with initial postoperative paralysis. Overall, 6 of these 7 patients (85.7%) with persistent facial nerve dysfunction had follow-up of less than 1 month. All transient and permanent facial nerve dysfunction was in the distribution of the marginal mandibular nerve only. No statistically significant predictors of complications were identified. CONCLUSIONS: We report acceptable but not insignificant rates of marginal mandibular distribution facial nerve injury, poor scarring, wound infection, and recurrence following resection of cervicofacial nontuberculous mycobacterial lymphadenitis in children that must be discussed with patients and parents before operation. No presentation or operative variables predicted the complications.

Airway management for intubation in newborns with Pierre Robin sequence.

OBJECTIVES/HYPOTHESIS: To review airway management in Pierre Robin sequence (PRS) newborns undergoing general anesthesia and to determine if endotracheal intubation is safe in this population. STUDY DESIGN: Case series and retrospective chart review at a tertiary children's hospital. METHODS: PRS newborns who underwent endotracheal intubation or other airway intervention before 3 months of age between January 2000 and July 2011 were identified from a pediatric otolaryngology practice database. Indications for airway intervention, anesthetic management, method of intubation, and comorbid conditions were collected. RESULTS: Thirty-three PRS newborns were identified. Twenty had isolated PRS, and 13 had PRS related to a coexisting syndrome. Thirteen of 35 (37%) endotracheal intubations performed in PRS newborns prior to mandibular distraction osteogenesis were accomplished with direct laryngoscopy. The remaining 22 of 35 (63%) who failed intubation with direct laryngoscopy were intubated over a flexible fiberoptic bronchoscope. No significant difference was observed between the isolated and syndromic PRS newborns with regard to technique utilized for intubation. No patient required rescue laryngeal mask airway or emergent tracheotomy, and no case resulted in death. CONCLUSIONS: This series demonstrates that endotracheal intubation is safe and effective in PRS newborns. In patients who failed intubation with direct laryngoscopy, intubation over a flexible fiberoptic bronchoscope provided a reliable alternative method. Although airway management in PRS newborns poses a significant challenge, experienced otolaryngologists and anesthesiologists can successfully manage these difficult airway cases.

A theoretical cause of nasal obstruction in patients with repaired cleft palate.

BACKGROUND: During cleft palate repair, mucoperiosteal flaps are elevated from the palatal shelves and the nasal septum to accomplish tension-free closure of the nasal floor. This study was designed to geometrically describe how palate repair inherently limits airflow by decreasing nasal cavity cross-sectional area and increasing nasal airflow resistance. In addition, this study investigates whether the width of the repaired cleft palate directly affects the degree of postoperative airflow resistance. METHODS: A simplified geometric model of normal nasal cavity anatomy was compared with an equivalent schematic representing an individual with an unrepaired palatal cleft. Mathematical equations approximating the cross-sectional areas of normal and cleft nasal cavities were created. The theoretical postoperative loss of nasal cavity cross-sectional area was then considered for both unilateral and bilateral palatal clefts. RESULTS: According to this geometric model, the cross-sectional area of the nasal cavity is decreased in patients who undergo cleft repair. Repaired bilateral clefts experience a greater area loss than their unilateral counterparts. CONCLUSION: Nasal cavity resistance is higher in patients who have undergone cleft repair than in individuals who have not undergone cleft repair. because tension-free closure of the nasal floor inherently reduces cross-sectional breathing area. The wider the cleft, the higher the resistance to nasal airflow postoperatively. This iatrogenic source of nasal resistance is likely additive to other anatomic contributors to airflow resistance observed in individuals with cleft nasal deformities.

Incidence of operative endoscopy findings in recurrent croup.

OBJECTIVE: Develop an evidence-based model for predicting operative endoscopy findings in patients with recurrent croup. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care children's hospital. SUBJECTS AND METHODS: Retrospective chart review was performed on 124 patients who received consultation for recurrent croup between 2000 and 2009. Direct laryngoscopy and bronchoscopy findings were categorized as normal, mildly abnormal (incidental findings or grade I subglottic stenosis), moderately abnormal (grade II subglottic stenosis), or severely abnormal (grade III-IV subglottic stenosis). RESULTS: Of 124 consultations for recurrent croup, 81 patients (average age 3.5 years) underwent operative endoscopy. Normal examinations occurred in 33 of 81 (41%). Abnormal findings were encountered with the following frequency: mildly abnormal, 40 of 81 (49%); moderately abnormal, 6 of 81 (7.5%); and severely abnormal, 2 of 81 (2.5%). Relative risk (RR) of either moderately abnormal or severely abnormal findings was increased for patients who had a history of previous intubation (RR = 9.8; P = .002), prematurity (RR = 6.4; P = .01), or inpatient consultation (RR = 5.3; P = .028). The rate of moderately or severely abnormal findings in patients without the risk factors of intubation and age younger than 1 year was 0 of 48 (0%; confidence interval, 0%-7.4%). Mild abnormalities in this group were encountered in 27 of 48 (56%). CONCLUSION: Mild airway abnormalities are common in children with recurrent croup and cannot be ruled out based on history. In the absence the risk factors of previous intubation, age younger than 1 year, or inpatient consultation, the incidence of a significantly abnormal finding is quite low. A predictive model based on this evidence is discussed.

Complete peripartum airway management of a large epignathus teratoma: EXIT to resection.

We present a case of a female infant that was diagnosed on prenatal ultrasound with an oral mass, and subsequently underwent a planned EXIT procedure to secure the airway. Ultimately the epignathus teratoma was excised at 3 days of age. The number of cases in which neonates have survived epignathus teratomas has increased in the past decade due to improved prenatal imaging and the development of the EXIT procedure. This case demonstrates the importance of a multidisciplinary approach that must be established when a prenatal diagnosis raises concern for airway compromise, and the thorough work-up required for definitive management.

Mandibular distraction osteogenesis in infants younger than 3 months.

OBJECTIVES: To examine the long-term outcomes and complications in infants with upper airway obstruction and feeding difficulty who underwent bilateral mandibular distraction osteogenesis (MDO) within the first 3 months of life and to identify any preoperative characteristics that may predict the long-term outcome following early MDO intervention for airway obstruction. METHODS: An institutional, retrospective medical chart review was performed. Inclusion criteria were bilateral MDO performed at an age younger than 3 months, with a minimum follow-up of 3 years. A quantitative outcome measures scale was developed, and patients were scored based on long-term postoperative complications as well as airway and feeding goals. Factors such as need for an additional surgical procedure were also considered. RESULTS: Nineteen children were identified as having undergone MDO before 3 months of age and having more than 3 years of follow-up data. The mean age at distraction was 4.8 weeks (range, 5 days-12 weeks); the mean length of follow-up was 5.6 years (range, 37-122 months). Of these 19 patients, 14 had isolated Pierre Robin sequence (PRS) and 5 had syndromic PRS. All patients with isolated PRS had a good or intermediate long-term result. Infants with comorbidities such as developmental delay, seizures, or arthrogryposis had the poorest outcomes. CONCLUSIONS: Bilateral MDO is a relatively safe and effective means of treating airway obstruction and feeding difficulty in infants with PRS. The effects of this procedure, which carries a relatively low morbidity, persist through early childhood in most patients.

Distraction osteogenesis of the mandible for airway obstruction in children: long-term results.

OBJECTIVE: To determine the long-term results of distraction osteogenesis of the mandible for upper airway obstruction in children with micrognathia. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care children's hospital. SUBJECTS AND METHODS: The records of a pediatric otolaryngology practice and tertiary children's hospital were searched for patients treated with bilateral mandibular distraction osteogenesis for upper airway obstruction. Patients were selected if greater than three years of follow-up data were available. Data were analyzed for airway and feeding outcomes, and long-term surgical complications were identified. RESULTS: Thirty-two patients met study criteria. Of the 11 patients who had tracheotomy prior to distraction, seven were decannulated after the procedure. Seventeen patients needed perioperative gastrostomy. Seven are now able to feed orally. Fifteen patients treated with mandibular distraction were able to avoid gastrostomy tube placement altogether. The complication of open bite deformity was experienced by nine patients (28%). Five of 32 patients (16%) had tooth malformation, tooth loss, or dentigerous cyst formation while an additional three patients (9%) had long-term facial nerve injury. Nineteen patients (59%) were under three months old at the time of their distraction. Only one of these patients (5.2%) required an additional distraction procedure. CONCLUSION: Long-term follow-up data on patients treated with mandibular distraction for upper airway obstruction show sustained airway improvement. Additionally, micrognathic children treated with distraction have improved outcomes in oral feeding with a relatively low rate of long-term complications. It remains important to follow these patients to monitor the need for secondary reconstructive procedures.

Complications and satisfaction with pediatric osseointegrated external ear prostheses.

OBJECTIVES: To determine the complication rate and the patient satisfaction of titanium osseointegrated ear implants for congenital microtia and anotia. To discuss external ear prostheses as a treatment option for children with microtia. STUDY DESIGN: Retrospective chart review of patient records from a tertiary pediatric otolaryngology practice. METHODS: The charts were reviewed for all pediatric patients who had undergone titanium implants for attachment of auricular prostheses from 2000-2006. Data collected included demographic information, congenital syndrome, number and size of screws placed, occurrence of skin reactions, presence of other complications, and length of time patients wore the prosthetic ear. RESULTS: Eight patients, nine ears, were implanted. Median age was 7 years old. 6/8 had oculoauriculovertebral (OAV) syndrome, and 2 had isolated microtia. All titanium screws achieved complete osseointegration, and no implants have failed. 5/27 (18%) of the screws contacted dura, but no patients exhibited CSF leak, meningitis, or other central complications. 3/9 ears developed skin complications at the abutment sites, and 1/9 required revision soft tissue surgery. All the patients without current skin complications wear their prosthesis full time during waking hours. CONCLUSIONS: Titanium osseointegrated ear prosthesis is a safe and useful procedure for children with microtia. It should be presented as an option for these patients, with low complication rates and high acceptance rates by the patients.

Platelet-rich plasma for pediatric tonsillectomy patients.

OBJECTIVES/HYPOTHESIS: To determine if placement of autologous platelet-rich plasma (PRP) on the tonsil beds at the time of tonsillectomy would decrease postoperative pain and complications. STUDY DESIGN: Double blind, randomized, prospective study. METHODS: Seventy children were recruited to participate in the study, ranging in age from 4 to 15 years. They were randomized to treatment (PRP) or control groups. The parents, patients, and nurses were blinded as to which group they were in. Postoperative pain scores were obtained using the FACES pain scale and recorded in a home diary. RESULTS: Seventy patients were recruited to the study, and 12 did not submit their diaries and were considered to drop out of the study. Demographic data were similar for both control and treatment groups. Median pain scores, medication use, days to normal diet, and office visits did not differ between the two groups. CONCLUSIONS: PRP applied once at the time of tonsillectomy does not improve postoperative pain or recovery in pediatric patients.

Airway interventions in children with Pierre Robin Sequence.

OBJECTIVE: To describe the interventions required for successful airway management in children with Pierre Robin Sequence (PRS). STUDY DESIGN: Case series. SUBJECTS AND METHODS: The records of both a cleft and craniofacial clinic and a pediatric otolaryngology clinic were searched, and all children with PRS were identified. Data concerning feeding interventions, airway interventions, and comorbid conditions were extracted. RESULTS: Seventy-four cases of PRS were identified. Thirty-eight of the 74 children required airway intervention other than prone positioning. Fourteen of these 38 were managed nonsurgically with nasopharyngeal airway and/or short-term endotracheal intubation, whereas the remaining 24 required surgical intervention. Eighteen of the 24 underwent distraction osteogenesis of the mandible, one underwent tracheostomy, and five underwent tracheostomy followed by eventual distraction. CONCLUSION: In our series, over 50 percent of children with PRS required an airway intervention. These were both nonsurgical and surgical. As otolaryngologists, we must be prepared for the challenges children with PRS may present and the interventions that may be necessary to successfully manage these difficult airways.

Resolving feeding difficulties with early airway intervention in Pierre Robin Sequence.

OBJECTIVES/HYPOTHESIS: To observe rates of gastrostomy tube (g-tube) placement in Pierre Robin Sequence (PRS) and to determine whether relieving airway obstruction solves feeding difficulties. STUDY DESIGN: All PRS referrals to a multidisciplinary cleft team for children at a tertiary pediatric hospital from January 1988 to June 2006 were retrospectively reviewed. METHODS: Patients were analyzed for occurrence of g-tube placement, neurologic disorders, and airway intervention including tracheotomy and mandibular distraction osteogenesis. RESULTS: Sixty-seven PRS patients were divided into two categories: 51 (76.1%) isolated PRS (iPRS) and 16 (23.9%) with additional disorders and syndromes (sPRS). Patients were then placed into two subgroups: those who received early airway intervention and those who received late or no airway intervention. Of the 51 iPRS children, 12 (23.5%) received early airway intervention, none of whom required a g-tube. There were 39 (76.5%) children who received late or no airway intervention, and 5 (12.8%) of these required g-tube placement. Of the 16 sPRS children, 8 (50%) received early airway intervention, and 7 (87.5%) of these still required a g-tube. Of the remaining 8 (50%) sPRS patients who received late or no airway intervention, 5 (62.5%) required a g-tube. CONCLUSION: In children with iPRS, feeding difficulties can be resolved with early airway intervention. Delaying airway intervention may necessitate feeding assistance because all of the iPRS children who required a g-tube fell into this category. The presence of additional disorders and syndromes further complicates treatment because most of the sPRS children required g-tubes regardless of airway intervention.

Tracheal agenesis in newborns.

OBJECTIVES/HYPOTHESIS: A series of three newborns with tracheal agenesis is described. The preferred methods of diagnosis, description of the clinical course, and a review of the pertinent embryology, associated anomalies, and clinical management are presented. STUDY DESIGN: A retrospective study of a clinical series of referred patients from 2002 to 2003 who were seen at a single institution. METHODS: Chart review for clinical course and pathological specimens was performed in all cases. Three patients were identified with tracheal agenesis. RESULTS: All three newborns died within 48 hours of birth. All of the children underwent emergency laryngoscopy and neck exploration. Gross and microscopic pathological examination was accomplished on all patients. CONCLUSION: Although tracheal agenesis is rare, it may be more common than previously thought. The diagnosis is not straightforward, and the prognosis is grim. The embryology of the trachea and other foregut derivatives is closely related, and associated birth defects are common.